[Dyserythropoietic syndromes: incidence, diagnosis, therapy].
The nosography of the dyserythropoietic syndromes remains poorly defined in the field of clinical hematology. The prominent pathophysiologic feature lies in the "ineffective erythropoiesis" as expressed by bone marrow erythroid hyperplasia with dysplasia accompanied by a normal or only slightly increased reticulocyte count. Both erythrokinetics and ferrokinetics are impaired, as shown by either slight reduction of the red cell survival or marked increased rate of serum iron transport together with reduced cellular iron utilization. The dyserythropoietic syndromes can be classified as acquired, secondary or congenital. The acquired ones, especially the sideroblastic forms, belonging to the myelodysplastic syndromes, are typical of the elderly whereas the congenital are of childhood. Their treatment is still a matter of controversy. However, the employment of folic acid, Vit. B12, pyridoxine and androgens can be useful in selected cases. In case of severe anemia, blood transfusion are required in association with iron chelating agents. However, some biological molecules, such as erythropoietin, interleukins 3 and 4, hemopoietic growth factors (especially GM-CSF), could represent future prospects of treatment.
Cacciola
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D000742:Anemia, Dyserythropoietic, Congenital / Q000175:diagnosis / Q000628:therapy; D000753:Anemia, Refractory / Q000175:diagnosis; D000756:Anemia, Sideroblastic / Q000151:congenital / Q000175:diagnosis; D003676:Deferoxamine / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D013577:Syndrome; D014815:Vitamins / Q000627:therapeutic use
D014815:Vitamins; D003676:Deferoxamine
Le sindromi diseritropoietiche: frequenza, diagnosi, terapia.